OBSERVATION One and One-half Syndrome With Supranuclear Facial Weakness Magnetic Resonance Imaging Localization

C. Alan Anderson, MD; Elliot Sandberg, MD; Christopher M. Filley, MD; Sally L. Harris, MD; Kenneth L. Tyler, MD

Objective: To provide clinicoanatomical correlation for Main Outcome and Results: Electromyographic a small pontine tegmental ischemic stroke producing the findings were consistent with supranuclear facial one and one-half syndrome associated with supra- involvement. The patient had nearly complete recov- nuclear facial weakness. ery after 1 year.

Design: Case report. Conclusions: To our knowledge, this is the first Setting: Tertiary care center. report of supranuclear facial weakness in association with the one and one-half syndrome. The location of Patient: A 70-year-old man developed left-sided the lesion provides evidence of the existence of corti- facial weakness sparing the forehead, a left inter- cofugal fibers that extend to the facial nucleus in the nuclear ophthalmoplegia, and a complete left horizon- dorsal paramedian pontine . tal gaze palsy immediately after percutaneous translu- minal coronary angioplasty. Magnetic resonance imaging demonstrated a small lesion in the left para- median aspect of the dorsal pontine tegmentum. Arch Neurol. 1999;56:1509-1511

HE ONE and one-half syn- REPORT OF A CASE drome consists of a horizon- tal gaze palsy (“the one”) A 70 year old man developed horizontal and an ipsilateral inter- diplopia immediately after percutaneous nuclear ophthalmoplegia transluminal coronary angioplasty. There (“theT one-half”).1 When the eye that is con- was no history of stroke or visual distur- tralateral to the lesion lies in a position of bance. His risk factors for atherosclerosis abduction at rest, the syndrome has been included hypertension, hyperlipidemia, called paralytic pontine exotropia.2 The hori- and obesity. zontal gaze palsy results from involve- The results of his general physical and ment of either the ipsilateral paramedian mental status examinations were nor- pontine or the ipsilat- mal. He had a complete horizontal gaze eral , and the inter- palsy to the left for voluntary, tracking, and nuclear ophthalmoplegia results from vestibulo-ocular movements. Conver- From the Departments of involvement of the ipsilateral medial lon- gence was impaired, with only minimal ad- Neurology (Drs Anderson, gitudinal fasciculus. We describe a man duction of both eyes. A left internuclear Sandberg, Filley, Harris, and with a small pontine tegmental ischemic ophthalmoplegia was present, with fail- Tyler), Surgery (Division of stroke, presumed to be embolic, whose ure of adduction in the left eye and hori- Emergency Medicine) clinical findings were limited to the one and zontal nystagmus in the abducting right (Dr Anderson), Radiology one-half syndrome and supranuclear fa- eye. In primary position, the patient had (Dr Sandberg), Psychiatry cial weakness. Patients previously de- resting exotropia of the right eye and was (Dr Filley), Medicine scribed as having facial weakness and the able to adduct the right eye to midposi- (Dr Tyler), Microbiology one and one-half syndrome all had a lower tion when using it for fixation. Vertical eye (Dr Tyler), and Immunology motor neuron pattern of facial weakness. movements and eyelid function were in- (Dr Tyler), University of Colorado School of Medicine, To our knowledge, this is the first report tact, and the pupils were briskly and sym- and the Denver Veterans Affairs of supranuclear facial weakness in associa- metrically reactive to light. He had left- Medical Center (Drs Anderson, tion with the one and one-half syndrome, sided facial weakness that involved the Sandberg, Filley, and Tyler), and the location of the lesion was con- muscles of the mouth and lower orbicu- Denver, Colo. firmed by magnetic resonance imaging. laris oculi, sparing the upper orbicularis

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©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 oculi and the muscles of the forehead (a central pattern of weakness), a diminished left corneal reflex, and a slight decrease in pinprick sensation on the left side of the face. The findings of the rest of the neurological examination were normal. All serum laboratory values were unre- markable. A magnetic resonance imaging scan obtained on hos- pital day 3 demonstrated a well-defined focus of in- creased signal character localized to the left paramedian aspect of the dorsal pontine tegmentum, just ventral to the (Figure 1). The lesion measured 5 ϫ 3 ϫ 6 mm. Diffusion-weighted images supported the acuteness of the finding. The remainder of the patient’s hospital course was uneventful. The results of his examination were un- changed 4 months later. The patient returned for follow-up after 1 year. He had no complaints and felt that he had returned to nor- mal. On examination, he had conjugate gaze in primary position and a subtle left internuclear ophthalmoplegia. When fatigued, he had a subtle left conjugate horizontal gaze paresis. He had very mild lower facial weakness and normal findings on his sensory examination. A second magnetic resonance imaging scan demonstrated resolu- tion of the abnormality in the . Electromyography of the showed no evidence of denervation. Blink reflex testing revealed R1 delay on the left, and bilateral R2 delay with stimulation of the left supraor- bital nerve, consistent with residual dysfunction of the trigeminal nucleus.

Figure 1. Axial fluid attenuated inversion recovery magnetic resonance COMMENT imaging scan (top) and sagittal reconstruction T2-weighted magnetic resonance imaging scan (bottom) show an area of increased signal Since Leo Tolstoy’s first account of the one and one-half consistent with an ischemic stroke in the left paramedian dorsal pontine syndrome resulting from a stroke,3 the eye tegmentum, adjacent to the fourth ventricle. movement disorder has been described both as an iso- lated syndrome and in association with dysphagia, dys- tract as a series of discrete bundles, decussating to reach arthria, hemiparesis, ataxia, hemisensory loss, and contralateral cranial nerve nuclei.6 Among the bundles involuntary movements.2,4 Causes of the syndrome he identified were a subthalamic bundle that extends to include multiple sclerosis, hemorrhage, tumors, and the nucleus of the third cranial nerve (Figure 2, fasth), ischemic stroke.4 The anatomical substrate for the syn- a peduncular bundle to cranial nuclei III, VI, and XI (Fig- drome, including involvement of the ipsilateral medial ure 2, fapd), a pontine bundle to cranial nuclei V, X, and longitudinal fasciculus and either the abducens nucleus XII (Figure 2, fap), and a pontomedullary bundle to the or the pontine conjugate lateral gaze center in the dor- nucleus of the that also contributes fibers to sal pontine tegmentum adjacent to the fourth ventricle, the pontine bundle (Figure 2, fabp). This fourth bundle was first proposed by Fisher1 and later confirmed by leaves the pyramidal tract at the level of the pontomedul- neuropathological and neuroimaging findings.4,5 The lary junction and passes through the pontine tegmentum onset of symptoms in our patient, immediately after an to reach the facial nucleus. The clinical significance of arterial angiographic procedure and without arrhyth- these bundles is that lesions selectively involving one mia, hypoxia, or hypotension, suggests an embolic bundle may produce a restricted pattern of supranuclear source for the stroke. The paramedian pontine tegmen- weakness. Prior reports have confirmed the presence of tum is irrigated by the long anteromedial group of para- supranuclear fibers extending to the facial nucleus in the median pontine perforating branches off the basilar pons,7 but the exact course of these fibers through the artery.5 pons is unknown. Some of the corticobulbar fibers The left supranuclear facial weakness in our patient project directly to the facial nucleus, and others connect is consistent with involvement of corticobulbar fibers via pontine reticular interneurons. There are also corti- that extend to the facial nucleus. The facial nucleus is in- cobulbar fibers that descend ipsilaterally in the ventro- nervated by fibers that originate in the precentral gyrus. medial part of the rostral medulla, decussate, and then Corticobulbar fibers, including those destined to reach ascend rostrally to the contralateral facial nucleus.8 We the facial nucleus, descend in the internal capsule in as- believe that our patient’s weakness resulted from in- sociation with the . Dejerine noted that volvement of the fourth bundle of Dejerine in the pon- in the brainstem corticobulbar fibers leave the pyramidal tine tegmentum.

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©1999 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 tient’s upper motor pattern of facial weakness was due NL 3 to partial damage to the intramedullary fascicles of the NL 2 NC seventh nerve, rather than to interruption of cortico- Cia NL Cirl bulbar fibers to the facial nucleus. However, lesions in- 1 Cip(Co-M) NC Th volving these fascicles typically produce a lower motor Ci(g) neuron pattern of facial weakness. We are unaware of a Ne (part ventrale) well-documented example of facial weakness due to a fas- Pul Co-N cicular lesion. The findings of electrodiagnostic testing VP { Co-M Rg Rm in our case showed no evidence of a lower motor neu- P RsTh Sgc ron lesion. One report also describes palatal myoclonus (III)fasth a Ln Qa in association with the one and one-half syndrome and fapd PLp Rm b facial weakness that is attributed to involvement of the (III,VI,XI) 11 { PLs c Qp IV adjacent central tegmental tract. III Flp SR IV The one and one-half syndrome with supranuclear (Vm,XII,XI-Xa)fap Vm FPoa facial weakness in our patient with a well-delineated, Vm small ischemic stroke of the paramedian pontine teg- Np Np VP VI Po FPop mentum represents another variant of this interesting CoM V4 syndrome.

(VII)fabp VII Accepted for publication May 24, 1999. IX Flp VI XII Reprints: C. Alan Anderson, MD, Neurology B-182, Py VII Xa IX University of Colorado Health Sciences Center, 4200 E 9th X+XI XI Ave, Denver, CO 80262. XII B XII CoN CoM Py REFERENCES NCp (XI,CII-IV)fab XI 1. Fisher C. Some neuro-ophthalmological observations. J Neurol Neurosurg Psy- Fa xPy P chiatry. 1967;30:383-392. Pa 1 CoM XI fcnc (XII,XI,CII-IV) 2. Sharpe J, Rosenberg M, Hoyt W, Daroff R. Paralytic pontine exotropia. Neurol- F1 P2 fcnd (CII-IV) Pc CoN ogy. 1974;24:1076-1081. F2 O1 pF OpR O M 3. Albin R. The death of Nicholas Bolkonski: neurology in Tolstoy’s War and Peace. 3 T 2 F3(c) 1 FPyc Arch Neurol. 1990;47:225-226. T O3 2 FPyd oF3 4. Wall M, Wray S. The one-and-a half syndrome: a unilateral disorder of the pon- T3 (XI,CII-IV)fab tine tegmentum: a study of 20 cases and review of the literature. Neurology. 1983; fPyh 33:971-980. 5. Kataoka A, Hori A, Shirakawa T, Hirose G. Paramedian pontine infarction. Neu- Figure 2. Dejerine’s drawing of the corticobulbar fibers, with the rological/topographical correlation. Stroke. 1997;28:809-815. corticospinal tract [Ci(g)] shown in red. The fibers to the facial nucleus leave the corticospinal tract in the fourth bundle (fabp) at the level of the 6. Puvanendran K, Wong PK, Ransome GA. Syndrome of Dejerine’s Fourth Reich. pontomedullary junction. The cortical origin of supranuclear fibers to the Acta Neurol Scand. 1978;57:349-353. facial nucleus is shown in the inset (CoM). For a full explanation of the 7. Hopf H, Tettenborn B, Kramer G. Pontine supranuclear facial palsy. Stroke. 1990; abbreviations in the figure, see reference 6. This is a schematic diagram. The 21:1754-1757. exact course of corticobulbar fibers in the brainstem is not known (from 8. Terao S, Takatsu S, Izumi M, et al. Central facial weakness due to medial med- Semiologie des affections du systeme nerveux. 2nd ed. Paris, France; ullary infarction: the course of facial corticobulbar fibres. J Neurol Neurosurg Masson et cie; 1926:201). Psychiatry. 1997;63:391-393. 9. Oommen K, Smith M, LaBadie E. Pontine hemorrhage causing Fisher one-and- a-half syndrome with facial paralysis. J Clin Neuroophthalmol. 1982;2:129-132. Previous cases of facial weakness with this eye move- 10. Eggenberger E. Eight-and-a-half syndrome: one-and-a-half syndrome plus cra- nial nerve VII palsy. J Neuroopthalmol. 1998;18:114-116. ment disorder describe a lower motor neuron pattern of 11. Wolin M, Trent R, Lavin P, Cornblath W. Oculopalatal myoclonus after the facial weakness, attributed to involvement of the facial one-and-a-half syndrome with facial nerve palsy. Ophthalmology. 1996;103: nucleus or fasciculus.9,10 It is conceivable that our pa- 177-180.

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